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Pediatric Neurology Quick Talks

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Title: PowerPoint Presentation Author: Matt Cross Last modified by: lindsay Created Date: 10/18/2005 4:13:28 PM Document presentation format: On-screen Show (4:3) – PowerPoint PPT presentation

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Title: Pediatric Neurology Quick Talks


1
Pediatric Neurology Quick Talks
  • Ataxia
  • Michael Babcock
  • Summer 2013

2
Scenario
  • 2 yo girl
  • Acute onset of not walking every since waking
    this morning
  • Crying and trying to be carried
  • No fever
  • No recent witnessed trauma
  • No erythema, no pain with palpation of
    extremities
  • Hx of normal development, walking for 8 months.
  • Awake and alert, but fussy

3
Refusal to Walk (or abnormal gait/limp)
  • Infections septic arthritis, osteomyelitis,
    discitis, myositis
  • Inflammatory transient synovitis
  • Trauma toddler's fractures, foot fracture,
    child abuse, contusions/bruises
  • Bony deformity/problem slipped epiphysis, hip
    dysplasia, asceptic necrosis (legg-calve-perthes),
    vaso-occlusive crisis, osteochondrosis, tumors.
  • Abdominal pain appendicitis, PID, abscess
  • Neurologic weakness (muscular dystrophy, NMJ,
    GBS, TM), ataxia, infections meningitis, spinal
    abscess.
  • Psychogenic - conversion

4
History
  • When was walking last normal onset of symptoms?
  • Any trauma?
  • Vitals? Fever?
  • Any LOC or abnormal movements?
  • nausea/vertigo/posterior fossa symptoms?
  • Why is walking altered? - pain, weakness,
    numbness, imbalance, can't say?

5
Neurologic Exam walk refusal/abnormal walk
  • MS
  • CN pupils, eye movements (abnormal movements,
    opsoclonus), CN 8 (ear exam, tinnitus, hearing
    loss) peripheral vestibular neuropathy/labrynthi
    tis
  • Motor strength weakness gt myopathy/NMJ/periph
    eral nerve/UMN
  • Reflexes absent? --gtGBS, increased?-gtUMN
  • Sensory uncommon reason in kids B12, tabes
    dorsalis, diabetic peripheral neuropathy
  • Cerebellar Rapid alternating movements
    (finger-tapping/alligator chomp, toe-tapping),
    Finger-nose-finger, heel-knee-shin, other
    rhythmic movements-clapping, also listen to
    speech.
  • Gait romberg, tandem think of different
    abnormal gait types.

6
This lecture is on Ataxia
  • Children can present with wide-based, ataxic gait
    OR with refusal to walk due to sense of
    imbalance.
  • Static ataxia present in resting state
  • Kinetic ataxia present only with movement
  • Dystaxia
  • Dysmetria can't control distance/speed/power
    leads to past-pointing
  • Dysdiadochokinesia can't rapidly
    switch/alternate movements leads to poor rhythm
    (hand/foot tapping), leads to poor rapid
    alternating movements.

7
Cerebellum
  • Midline vermis
  • Dysarthia
  • Truncal titubation
  • Gait abnormalities
  • Hemispheres
  • Double-crossed tracts, ipsilateral findings
  • Limb dysmetria
  • Ipsilateral veering while walking
  • Tremor
  • hypotonia

8
Abnormal Gait
  • Spastic gait
  • Sensory ataxic gait wide-based, high-stepping,
    romberg
  • Cerebellar ataxic gait titubation, wide-based
  • LMN distal weakness, foot drop, high-stepping,
    but narrow-based
  • Myopathic proximal weakness, difficulty rising
    from chair, Gower's
  • Movement disorder gaits adventitial movements,
    lurching gait
  • Psychogenic Astasia-abasia wide variation
    with complex movements, often wide movement off
    vertical axis without falling.

9
Romberg test
  • -Sensory ataxia
  • -Pt can stand with feet together with eyes open,
    but not eyes closed.
  • -Cerebellar ataxia
  • -Pt can not stand with feet together even with
    eyes open.
  • -Positive romberg imbalance with eyes closed
  • -Testing for sensory ataxia.

10
Causes of ataxia
  • Intermittent
  • Benign paroxysmal vertigo
  • Seizure
  • Inborn Error of Metabolism
  • Basilar migraine
  • Chronic
  • Congenital anomaly of posterior fossa
  • Dandy-walker, chiari, cerebellar dysplasias
  • Hereditary ataxias
  • Freidrich ataxia, ataxia-telangectasia,
    Roussy-Levy, spinocerebellar ataxia, episodic
    ataxias
  • Neurodegenerative
  • abetaliproteinemia, vitamin E deficiency, Refsum,
    Niemann-Pick,
  • ACUTE
  • Brain tumor
  • Neuroblastoma
  • Head-trauma
  • Vertebrobasilar dissection/stroke
  • ADEM
  • Opsoclonus-Myoclonus-Ataxia
  • Acute Cerebellar Ataxia
  • GBS
  • Tick paralysis
  • Infections encephalitis, cerebellar abscess,
    ccute labrynthitis
  • Toxicities alcohol, lead, AED
  • Conversion reaction

11
Work-up for acute ataxia
  • Head imaging if AMS, focal neurologic signs,
    cranial neuropathies, marked asymmetric ataxia,
    trauma, other concerns for mass lesion
    posterior fossa symptoms/ elevated ICP symptoms
  • MRI is best, especially for posterior fossa
    lesions, demyelinating
  • CT if concern for hydrocephalus, trauma, mass
    lesions, unstable
  • Labs UDS, alcohol level, BMP (glucose), CSF if
    concern for infection or GBS. Metabolic
    evaluation for IEM if needed

12
Acute Cerebellar Ataxia
  • Age 2-5 yo
  • Preceded by febrile illness 4 days to 3 weeks
  • Symptoms acute, maximal at onset gait
    disturbance is most common, can also have motor
    symptoms/fine tremor, dysarthria, 50 have
    nystagmus
  • Some with vomiting and headache in older children
  • Should NOT have fever, meningismus, seizure, AMS,
    focal neurologic signs, insiduous onset
  • Differential toxic/metabolic,
    infectiousmeningitis, encephalitis, ADEM,
    labrynthitis, structural, metabolic,
    neurodegenerative, episodic ataxic syndrome.
  • Work-up CSF if fever, meningitic signs, AMS
    imaging if trauma, ICP signs, posterior fossa
    signs, asymetric focal findings, ADEM
  • Usually resolves within 2-3 weeks if worsening
    or relapse than reconsider diagnosis
  • Tx sometimes steroids, IVIG in
    severe/refractory cases
  • 10 will have some long-term effects, but most
    recover completely
  • older age and EBV associated with worse prognosis.

13
Opsoclonus-Myoclonus-Ataxia Syndrome
  • Dancing eyes dancing feet
  • Mean age 2 yo.
  • Opsoclonus uncontrolled, frequent, conjugate,
    saccidic movement of eyes in all directions.
  • irritability, sleep problems
  • Auto-immune Paraneoplastic neuroblastoma
    (unknown Ab) parainfectious
  • Differential other causes of myoclonus, ataxia
    toxic/metabolic dz, structural
  • Work-up Look for neuroblastoma CAP MRI,
    VMA/HVA, MIBG scan. If no neuroblastoma, then
    MRI brain to look for structural,
    toxic/metabolic, med review, infections viral
    (hep C, Lyme, EBV, HIV, coxsackie, rota,
    mycoplasma, GAS).
  • Tx - Treat movements with immunosuppression-
    steroids, IVIG, rituximab.

14
Freidrich Ataxia
  • AR
  • Most common hereditary ataxia 150,000
    Caucasian
  • Frataxin gene 9q13 trinculeotide repeat
  • Affects brain, heart, pancreas. Slow onset and
    progressive.
  • Onset before age 25 (often by age 5), ataxia of
    all 4 limbs, cerebellar dysarthria, absence of LE
    reflexes, pyramidal signs (weakness, extensor
    plantars), optic atrophy, brainstem involvement
    (swallowing dysfunction), early loss of
    position/vibration without pain/temperature loss,
    sensineuronal hearing loss
  • Cardiomyopathy 66 of patients hypertrophic
    main cause of death is arrhythmia and heart
    failure
  • Diabetes Mellitus 33 of patients

15
Ataxia-telangectasia
  • AR ATM gene 11q22.3
  • 120-100,000
  • ATM gene involved in detecting DNA damage, plays
    role in cell cycle progression
  • Neurologic effects
  • Progressive cerebellar ataxia (earliest feature
    children usual begin walking, then have problems
    with fluid walking) articulation problems
  • abnormal eye movements oculmotor apraxia is
    earliest
  • Can also have dystonia, chorea, peripheral axonal
    neuropathy
  • Oculocutaneous telangectasias oculobulbar,
    nose, face, ear, neck. Appear age 3-5 yo. Also
    can have cafe-au-lait macules.
  • Immune deficiency recurrent sinopulmonary
    infections
  • Pulmonary disease major cause of death
    recurrent infections, interstitial lung disease,
    neuromuscular abnormalities (dysphagia,
    aspiration, resp muscle weakness)
  • Malignancy 10-20 will develop cancer.

16
PREP Question
A 4 year old boy presents to the emergency
department with balance problems. He had been
previously healthy, but his walking has worsened
progressively for the past 2 days, with
staggering and lurching. On PE, the boy is
cooperative and alert. His muscles are not
tender, and his joints are not red, swollen or
tender. His vision seems functionally normal, but
there is end-gaze nystagmus in all directions.
When sitting independently, his head and trunk
bob. His strength appears normal, and his
reflexes are normal. When asked to stand with his
hands outstretched, a symmetric tremor is
evident, and worsens as he approaches this target
on finger-to-nose testing. His gait is
broad-based. A urine toxicology screen reveals
normal results. Brain magnetic resonance imaging
shows no tumors or other gray or white matter
lesions. Lumbar puncture shows 3 WBCs, 2 RBCs,
protein 20, glucose 50.
  • Of the following, you are MOST likely to advise
    the child's mother that
  • A. Intravenous steroids significantly reduce
    recurrence risk
  • B. Neuroblastoma is a common cause of these
    symptoms
  • C. Repeat lumbar puncture is needed in 2 days
  • D. Symptoms may resolve in weeks to months
  • E. Symptoms usually resolve after antibiotic
    treatment

17
D. Symptoms may resolve in weeks to months
  • -Cerebellar ataxia
  • -Cerebellar findings on exam Lateral end gaze
    nystagmus, dysmetria, ataxia, titubation.
  • -Subacute onset with normal mental status
    suggests acute cerebellar ataxia
  • -Usually acquired after infection or immunization
  • -MRI shows lesion
  • -Regarding other choices
  • A. Intravenous steroids significantly reduce
    recurrence risk They don't
  • B. Neuroblastoma is a common cause of these
    symptoms not as common
  • C. Repeat lumbar puncture is needed in 2 days no
    new information
  • E. Symptoms usually resolve after antibiotic
    treatment Not a primary infectious process

18
References
  • -Uptodate articles
  • http//www.studyblue.com/notes/note/n/pnb-exam-3/d
    eck/107773
  • http//www.medicinenet.com/image-collection/ataxia
    _telangiectasia_ear_picture/picture.htm
  • -maria, b. current management in child
    neurology.2009
  • -http//www.78steps.com/examination-sequence/arm-d
    rift.html
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